A case of hemophagocytic lymphohistiocytosis caused by an Epstein-Barr virus infection, presenting with unremitting fever and rash
Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by excessive activation of lymphocytes and macrophages, leading to cytokine storm. Infection-associated HLH is most common, and Epstein-Barr virus is the leading triggers. Quick diagnosis is essential for starting the tr...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
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Korean Society of Pediatric Emergency Medicine
2019-06-01
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| Series: | Pediatric Emergency Medicine Journal |
| Subjects: | |
| Online Access: | http://pemj.org/upload/pdf/pemj-2018-00297.pdf |
| Summary: | Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by excessive activation of lymphocytes and macrophages, leading to cytokine storm. Infection-associated HLH is most common, and Epstein-Barr virus is the leading triggers. Quick diagnosis is essential for starting the treatment before irreversible damage. We report a case of 16-year-old boy who presented with unremitted fever, jaundice, and erythematous maculopapular rash all over the body. Investigations showed thrombocytopenia, hyperferritinemia, hypertriglycemia, and the bone marrow biopsy showed hemophagocytosis. Epstein-Barr virus antibody was positive. He responded to chemotherapy as per the HLH-2004 protocol and supportive treatment, and was discharged without complication on day 17. |
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| ISSN: | 2383-4897 2508-5506 |